Purpose of review The purpose of this review is highlighting the most recent evidence on the clinical efficacy and toxicity of antimalarials in systemic lupus erythematosus (SLE). Recent findings New data confirm the effects of antimalarials in preventing SLE activity, damage and infections and in decreasing mortality. An important reduction in use of health resources is related to continued antimalarial use. Hydroxychloroquine (HCQ) may prevent preeclampsia in pregnant women with SLE. HCQ ocular toxicity is infrequent and could be associated with blood levels. Gastrointestinal and skin toxicity are underrecognized and could influence adherence. Prolongation of QT interval is extremely unusual with HCQ. Doses of HCQ of 200 mg/day seem to offer a good efficacy/toxicity balance. HCQ protection against herpes zoster and Pneumocystis jirovecii infection has been shown. On the contrary, HCQ prescription by doctors and adherence by patients are both under recommended standards. The recent coronavirus disease 2019 pandemic has resulted in a significant shortage of HCQ in many countries with possible consequences in the correct treatment of lupus patients. Summary Recent evidence reinforces the central role of HCQ in SLE therapy. The reduction in activity, damage accrual and mortality is consistent across studies, countries and ethnical groups. On the contrary, and despite the well established beneficial effects of prolonged regular HCQ therapy, many SLE patients do never take this drug or it is eventually stopped in the setting of severe flares, pregnancy or presumed toxicity. Every effort must be made to assure the correct prescription of HCQ and not to withdraw the drug unless unequivocal signs of toxicity are present.

OBJECTIVE To compare the influence of antiphospholipid antibodies (aPL) on global and cardiovascular damage in patients with systemic lupus erythematosus (SLE) diagnosed before and after year 2000. METHODS 286 patients from the Lupus-Cruces cohort with a minimum follow-up of 5 years, divided into two sub-cohorts according to the date of diagnosis, before 2000 (<2000) and from 2000 on (≥2000). We compared the mean SDI score and global and cardiovascular damage-free survival rates in the presence/absence of aPL in both sub-cohorts. Variables potentially modulating damage among aPL-positive patients were analysed. RESULTS The sub-cohorts were comparable for demographic and lupus-related variables except for treatment variables: the ≥2000 sub-cohort received lower doses of prednisone and more hydroxychloroquine, low-dose aspirin, statins, immunosuppressive agents and Vitamin D. aPL-positive patients in the <2000, but not in the ≥2000 sub-cohort, accrued more damage compared with aPL-negative. In the <2000 sub-cohort, the adjusted HRs for global and cardiovascular damage in aPL-positive vs. aPL-negative patients were 1.98 (95% CI 1.24-3.14) and 9.3 (95% CI 3.24-26.92), respectively. No differences in damage were seen between aPL-positive and aPL-negative patients in the ≥2000 sub-cohort. Hypertension (HR 4.64, 95%CI 1.33-16.19), lupus anticoagulant (HR 3.85, 95%CI 1.1-13.41) and the number of months on hydroxychloroquine (HR 0.97, 95%CI 0.95-0.99) were independent predictors of vascular damage in the combined analysis of all aPL-positive patients. CONCLUSION The effects of aPL on damage accrual in SLE patients have been reduced over the last years. The widespread use of hydroxychloroquine and a better thromboprophylaxis are likely causing this change.

BackgroundAntihistone antibodies (AHA) have been linked to Drug-Induced Lupus Erythematosus (DILE) for decades1. However, for some authors this relationship is not so clear and sugest that the presence of these autoantibodies is related to other autoimmune diseases more frequently2,3,4.ObjectivesThe main objetive of this work was to study the association of AHA with different autoimmune entities (including DILE) and secondarily, look into which clinical manifestations and which autoantibodies are more frequently related to AHA.MethodsWe performed a descriptive study. A database was constituted using all patients with AHA+ in any blood analysis between years 2000 and 2016 in the University Hospital Complex of Vigo. The variables of the study were: presence of autoimmune disease, clinical manifestations and related autoantibodies.ResultsVariableMenWomenAll% Total Age504546Gender155873100SLE8273548DILE0000Scleroderma0445Sjögren18912Rheumatoid Arthritis0334No diagnosis5192433Malar rash1111216Photosensitivity2111318Oral ulcers1101115Arthritis6313751Lupic nephropathy4131723Raynaud1111216Hematological abnormalities5202534AntiRo+4121622AntiLa+25710AntiSm+381115AntiDNAds+3252838None of the 73 patients AHA+ developed DILE while almost the 50% of them suffer any other autoimmune disease. We found a high percentage of AHA+ patients with lupus erythematous complications such as arthritis and hematological abnormalities. AntiDNAds antibody was the more frequent coexpressed autoantibody.Conclusions AHA detection is not useful as DILE screening.AHA+ sugest the presence of other autoimmune disease rather than DILE.AHA+ may be related to lupus erythematous systemic complications. References Fritzler MJ, Tan EM. Antibodies to histone in drug-induced and idiopathic lupus erythematosus. J Clin Invest. 1978;62:560–567.Peng SL, Craft J. Antinuclear antibodies. In: Ruddy S, Harris ED, Sledge CB, eds. Kelley's Textbook of Rheumatology. 6th ed. Philadelphia, Pa: WB Saunders; 2001:166.Kubo M, Ihn H, Yasawa N, et al. Prevalence and antigen specificity of antihistone antibodies in patients with polymyositis/dermatomyositis. J Invest Dermatol. 1999;112:711–715.Zirwas MJ, Kress DW, Deng JS. The Utility of Antihistone Antibody Screening in the Diagnosis of Drug-Induced Lupus Erythematosus. Arch Dermatol Apr 2004; VOL 140: 494–495. Disclosure of InterestNone declared

BackgroundAntiphospholipid syndrome (APS) is a multisystem disease, and the heart may be affected from 10 to 50% in some series [1,2]. Heart valve lesions are the most frequent manifestations. Coronary arteries and myocardium may be also affected. Other findings include chronic thromboembolic pulmonary hypertension and accelerated atherosclerosis. Some studies have suggested a higher incidence of cardiac involvement in patients with secondary APS or those with higher titers of antiphospholipid antibodies. However, cardiac involvement in APS are not yet well known because there are few published series and most of them have a short-term follow-up.ObjectivesThe aim of our study was to determine the prevalence and the type of cardiac involvement in patients with APS. Also, risk markers of cardiac involvement in these patients were attempted to identify.MethodsA case series of patients with APS diagnosed and followed for a long period in a specific unit of Thrombosis and Vasculitis was conducted. Clinical and laboratory data were collected in a retrospective manner. To attempt to identify risk markers of cardiac involvement, patients with cardiac manifestations were compared with patients without heart involvement. Chi2 and t-student were used, using the statistical package SPSS18.0.Results32 patients with APS were included, 21 (66%) female, mean age 64.3±16.44 years [range 34-84], with a mean follow-up of 9.2 years (median 7 years). 8 patients (25%) had a secondary APS: 3 SLE, 2 Sjögren's syndrome, 1 rheumatoid arthritis, 1 systemic sclerosis and 1 demyelinating disease. 4 (13%) were pure obstetric APS, 9 (28%) had an arterial thrombosis, 9 (28%) had a venous thrombosis and 10 (31%) had mixed events (arterial, venous, and obstetric). 23 (72%) of patients had a cardiovascular risk factor (hypertension, dyslipidemia, type 2 DM). 19 patients of 32 with APS (59%) had cardiac involvement. 15 patients (79%) had valve disease (7 mitral valve, 3 aortic valve and 5 both mitral and aortic valves), and 8 (26%) had a coronary ischemic event. No patients had pulmonary hypertension. The comparison results between the two groups are shown in the following table.Table 1Cardiac involvementNon-cardiac involvementStatistical(n=19)(n=13)significanceAge in years (mean ± SD)69.4±14.7059.9±16.53p=0,03*Female68%62%p=0.8Cardiovascular risk factors79%62%p=0.2Secondary APS21%31%p=0.6Pure obstetric APS11%15%p=1Lupus anticoagulant42%46%p=0.9aβ2GPI or anticardiolipin Abs79%46%p=0.1IgG anticardiolipin33.3±14.2923.1±13.29p=0.6IgM anticardiolipin20.3±3.9018.9±8.22p=0.8IgG aβ2GPI20.5±12.7016.2±6.38p=0.7IgM aβ2GPI15.9±10.307.0±2.49p=0.4ConclusionsMore than half of patients with APS had cardiac involvement. Heart valve disease was the most frequent manifestation, almost always mitral valve. No patients had pulmonary hypertension. The only risk marker of cardiac involvement in our series was the age; patients with cardiac involvement were 10 years older on average.ReferencesCardiac involvement in the antiphospholipid syndrome. Tenedios F et al. Lupus 20...

BackgroundSystemic sclerosis (SSc) is a connective tissue disease of unknown origin, with heterogeneous clinic presentation and chronical and progressive evolution. There is a subtype of SSc without cutaneous presentation designated as systemic sclerosis sine scleroderma (ssSSc) that is difficult to diagnose with a delay on it producing worse prognosis.ObjectivesThe objective of this study was analyzing demographic features, clinical presentations, the treatment and evolution of ssSSc patients from our hospital.MethodsThere were analyzed in a retrospective way the patients diagnosed of SSc in our center between 1990 and May 2015, selecting those with ssSSc.ResultsAmong the 106 patients diagnosed of SSc in this period, only 5 patients (4.7%) had ssSSc. All of them were women with an average of 51 years. 3 patients (60%) had additionally an autoimmune hypothyroidism, other a polyglandular autoimmune syndrome and the other one had no autoimmune disease. Only one patient (20%) was HTA, another was smoker and another one had dyslipidemia. None of the 5 patients had suffered exposure to toxic elements. Average time delay for diagnosis was 10 years. For 3 patients initial diagnosis was in one case primary Raynaud phenomenon, other case pulmonary arterial hypertension (PAH) essential and in another one polyglandular autoimmune syndrome. 2 of the ssSSc diagnosis were done in 2006 (coincident with the opening of our monographic office of autoimmune systemic diseases). The reason for consultation in 4 of the patients (80%) was Raynaud phenomenon (RP) that was present in all of the patients during the tracking. Only one patient (20%) had digital ulcers, 2 patients (40%) esophagitis and 1 tendinopathy. 1 patient (20%) had PAH confirmed by right coronary angiography. All patients had antinuclear antibodies (ANA) positive, 80% anticentromere. A patient presented positivity to Anti Ro and another one to anticardiolipin antibodies. Nailfold capillaroscopy was made to 4 patients (80%), being all of them pathologic (Cutolo's pattern 3 active and 1 early). Spirometry was made in 4 patients (80%) founding diffusion alteration in 3 of them. The transthoracic echocardiogram performed to all patients was pathologic in the 3 of them (60%) (1 patent ductus arteriosus, 1 PAH and 1 valvular heart disease). Regarding treatment, 3 patients (60%) were treated with calcium antagonists and 2 (40%) received bosentan, without presenting none of them any serious adverse effect. It is important to highlight that 2 patients (40%) developed limited scleroderma after 9 and 4 years of diagnosis respectively. The patient with PAH died during the tracking because of right heart failure.ConclusionsssSSc diagnosis is difficult due to the absence of the main presentation, the cutaneous one. The most frequent beginning symptom is the RP, so including this symptom in the anamnesis and stablishing screening measures in patients with RP with detection of ANAs and periungual capillaroscopy is important for an early detection to allow diagnosis improvement.Referenc...

BackgroundGiant cell arteritis (GCA) is a vasculitis affecting large vessels and it is the most frequent type in patients over 50 years old. Although glucocorticoids (GC) still remain the mainstay of treatment in all these cases there is no yet clear evidence about optimal initial dose, regimen and relapses predictors and treatment [1, 2].ObjectivesTo assess the characteristics of treatment, relapse and outcomes of a biopsy-proven GCA patients and to compare these items between groups based on initial oral prednisone dose.MethodsWe performed a retrospective review of all patients diagnosed of biopsy-proven GCA in our institution (Complejo Hospitalario Universitario de Vigo) between 1 January 2000 and 31 December 2014. Additionally, they fullfilled at least 2 of other 4 1990 ACR classification criteria. Epidemiological, clinical and laboratory data, treatment and outcome of all these patients at diagnosis and during follow-up were analysed. We also compared groups by initial oral prednisone dose (≤40mg/day vs >40mg/day) using Cox models.ResultsDuring study period 70 patients were identified and analysed. Mean age at diagnosis was 77±7 years old and fifty-one (73%) were female. Charlson index was ≤1 in 83% of cases. Most frequent presenting symptoms were headache (87%), polymyalgia rheumatica (57%) and jaw claudication (48.6%). Ischaemic manifestations at diagnosis were seen in ten patients (14.3%). Median ESR and CRP was 88±26mm/h [31–140] and 92±88mg/dL [4–326] respectively. Median delay time from first symptom until diagnosis was 2 months [0–72]. We also analysed patients divided in two groups based on initial oral predisone dose (≤40mg/day [17 patients] vs >40mg/day [53 patients]). No differences was seen in sex, age, comorbidities (including cardiovascular risk factors), antiagregants/ immunosupressive therapy use or laboratory markers. Nevertheless prednisone dose at first month (30.2mg/day vs 48.6mg/day; p=0.001), time to reach ≤5mg/day of prednisone (11.8 vs 16 months; p=0.045) and total prednisone dose (7.2 vs 11.5 grams; p=0.001) were significantly higher in >40mg/day group although we did not see more GC-related adverse effects in this group (p=0.38). During follow-up period (median 29 months [3–140]) 18 patients had at least one relapse, however we did not observe statiscal differences between both groups (18% in ≤40mg/day vs 28.8% in >40mg/day; p=0.35). Time to first relapse (12±11.3 months in ≤40 vs 16±12.9 months in >40; p=0.67) and total treatment time (28±23.5 in ≤40 vs 35±25.1 months in >40) were similar in boths groups. We could not identified any factor that was related to relapses in our multivariate analysis. Twenty-two patients died during follow-up period but none were related with GCA.ConclusionsHigh-dose prednisone was the most frequent initial treatment of GCA patients.Lower doses regimen could be as safe and effective as the high-dose regimen.Relapses were seen in ¼ of patients but we could not identified any factor associated with them.ReferencesSalvarani C, Cantini F, Hunder GG. Polymya...

No abstract

No abstract